In the absence of hydrops or sepsis, a pericardial effusion is a rare occurrence in the neonate. We report a case of a neonate with a pericardial effusion in which there was an associated intracardiac hemangioma. Our literature review found 32 cases of pericardial effusion without hydrops in infants under 3 months of age; twelve of these cases were associated with intracardiac and pericardial tumors; 20 others were discovered to be randomly associated with other problems.